Abstract
BackgroundSystemic lupus erythematosus (SLE) is a complex multi-system disease, characterized by both autoimmune and autoinflammatory clinical and laboratory features. The role of type I interferon (IFN) in SLE has been demonstrated from the 2000s, by gene expression analyses showing significant over-expression of genes related to type I IFN signalling pathway (IFN signature). However, several studies questioned the role of measuring the intensity of IFN signature (IFN score) to chase SLE activity.We would assess if the IFN signature can help the clinical and therapeutic stratification of patients with pediatric SLE.MethodsWe measured the IFN score in peripheral whole blood from a series of subjects with childhood-onset SLE and correlated the results with clinical and laboratory parameters.ResultsThirty-one subjects were included in the study, among which the 87% displayed a positive IFN score. The only significant relation was found for high IFN score in subjects with normocomplementemia. No correlation was observed between IFN score and SLEDAI-2K, BILAG-2004 and SLICC. Patients with high IFN score and normal complement levels also presented lower anti-dsDNA antibodies.ConclusionsThe integration between IFN signature analysis and complement levels may easily distinguish two groups of subjects, in which the autoimmune or autoinflammatory component of the disease seems to be prevalent.
Highlights
Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a multisystem inflammatory and autoimmune disorder and it is characterized by a wide spectrum of clinical manifestations
Childhood-onset systemic lupus erythematosus [3] occurs before 18 years of age and presents a more severe clinical course than SLE developed in adult age [4, 5]
This study aims at assessing if the IFN signature analysis can contribute to identify subgroups of patients with paediatric SLE who may benefit from distinct treatments, based on the prevalent mechanisms involved in the disease pathogenesis
Summary
Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a multisystem inflammatory and autoimmune disorder and it is characterized by a wide spectrum of clinical manifestations. Significant progress has been achieved in the understanding of SLE, thanks to the identification of novel genetic variants associated with the disease, studies on murine models or findings involving gene expression and epigenetics. Genetic alterations leading to impaired function of the machinery devoted to clear apoptotic cells, waste nucleic acids and immune complexes play a significant role in both Mendelian and sporadic forms of SLE. The imbalance of these mechanisms results in the production of autoantibodies and in a dysregulated activation of inflammatory phenomena dominated by the secretion of type I interferons. Systemic lupus erythematosus (SLE) is a complex multi-system disease, characterized by both autoimmune and autoinflammatory clinical and laboratory features.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
